Dermatomyositis presenting with severe subcutaneous edema. Five additional cases and review of the literature

Milisenda, José César; Doti, Pamela; Cardozo, Celia; Moreno-Lozano, Pedro; Jerez, A.; Losno, Ricardo A.; Prieto, Sergio; Grau, Josep M.

doi:10.1016/j.ejim.2013.08.462

Cited by 7 publications

(13 citation statements)

References 0 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Subcutaneous edema was strongly associated with vascular lesions, especially capillary damage, which led to a marked decrease in serum albumin levels [27]. Prevalence of microinfarcts in muscle was higher in DM with subcutaneous edema [13], suggesting underlying vasculopathy.…”

Section: Discussionmentioning

confidence: 99%

“…Multisystem vasculopathy in DM comprises myocardial infarction, ischaemic stroke [8,9], renal infarction [10], retinopathy [11], digital infarcts [12] and microinfarcts in the muscles [13], indicating the involvement of vessels larger than capillaries. The significantly higher frequency of microinfarcts in edematous (80%) than in non-edematous (34%) adult DM suggests a prominent role of ischaemia and vasculopathy in the underlying edema [13,14]. Marked muscle ischaemia, focal punched-out vacuoles in myofibers and obvious capillary loss are observed in anti-NXP-2-positive DM [15].…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Perimysial microarteriopathy in dermatomyositis with anti‐nuclear matrix protein‐2 antibodies

Liu

Zheng

Gang

et al. 2019

Euro J of Neurology

View full text Add to dashboard Cite

Background and purpose Dermatomyositis (DM) with anti‐nuclear matrix protein‐2 (NXP‐2) antibodies usually shows multifocal ischaemic lesions in muscle. Here, we aimed to investigate the microarteriopathy underlying muscle ischaemia in anti‐NXP‐2‐positive DM. Methods A total of 16 patients diagnosed with anti‐NXP‐2‐positive DM were investigated by muscle biopsy. A total of 13 patients with DM with other myositis‐specific antibodies and 11 normal controls were included for comparison. Immunofluorescence assays were performed to localize endothelial cells, smooth muscle cells and pericytes, and to determine lesions in myofibers and microvessels by vascular endothelial growth factor and myxovirus resistance protein A (MxA). Electron microscopy was carried out to assess ultrastructure alterations. Results Subcutaneous edema, severe muscle weakness and dysphagia together with elevated creatine kinase, D‐dimer and triglyceride levels, and decreased albumin levels were found in anti‐NXP‐2‐positive DM. Muscle ischaemia included regional muscle edema, perifascicular atrophy, microinfarcts and focal punched‐out vacuoles. The density of arterioles was higher in anti‐NXP‐2‐positive DM (P ＜ 0.05). Perimysial arterioles with thickened vascular wall, thrombosis and lipid accumulation were found in the vascular wall of diseased perimysial arterioles. The frequency of diseased arterioles and thrombosis was higher in anti‐NXP‐2‐positive DM (P < 0.05). Sarcoplasmic vascular endothelial growth factor and MxA expression was observed in multifocal ischaemic lesions. MxA was present in endothelial and smooth muscle cells of the diseased arterioles and pericytes. Electron microscopy confirmed damaged capillaries and tubuloreticular structures. Conclusions Our research suggested that perimysial arterioles were most commonly involved in anti‐NXP‐2‐positive DM, which led to muscle ischaemia.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Perimysial microarteriopathy in dermatomyositis with anti‐nuclear matrix protein‐2 antibodies

Liu

Zheng

Gang

et al. 2019

Euro J of Neurology

View full text Add to dashboard Cite

show abstract

“…cases are an explosive onset, defined in days to weeks rather than years, and an increased prevalence of dysphagia in the subgroup with dermatomyositis (13 of 21 cases). Although a recent case series 9 reported an overrepresentation of pre-existing or newly diagnosed malignancy in their cohort (four of five cases), this is not a typical feature of anasarca-associated myositis. While a number of cases was reported prior to the availability of myositis-specific antibody detection, another striking feature is seronegativity in all reported cases.…”

Section: Brief Communicationsmentioning

confidence: 72%

“…To date, there have been 27 case descriptions of profound subcutaneous oedema as a primary manifestation of dermatomyositis (21 cases) and polymyositis (6 cases). [2][3][4][5][6][7][8][9][10][11][12] These have been reviewed elsewhere. 9,10 It has been proposed that these cases represent an aggressive variant of inflammatory myopathy with a relatively homogenous clinical phenotype characterised by abrupt onset, marked elevation in muscle enzymes and a male predilection.…”

Section: Discussionmentioning

confidence: 99%

Prominent subcutaneous oedema as a masquerading symptom of an underlying inflammatory myopathy

Anantharajah

Vucic

Tarafdar

et al. 2017

Internal Medicine Journal

View full text Add to dashboard Cite

The inflammatory myopathies are a group of immune-mediated inflammatory muscle disorders that typically present with marked proximal muscle weakness. We report four cases of inflammatory myopathies with marked subcutaneous oedema as their main complaint. Three of the four patients had normal or low levels of creatine kinase, an enzyme often markedly elevated in these disorders. Magnetic resonance imaging of the muscles, followed by a muscle biopsy were used to make a definitive diagnosis.

show abstract

“…Another hypothesis suggests that microischemia-producing microinfarction may play a role in the pathophysiology of the generalized edema. 13 Interestingly, in adults with dermatomyositis with generalized edema, symptoms evolve much quicker (2-8 weeks) than typical dermatomyositis cases. 13 This is similar to our patient, as her symptoms evolved over a period of only 2 weeks.…”

Section: Discussionmentioning

confidence: 99%

A 5-Year-Old Girl with Fatigue, Diffuse Edema, and Weakness

Barsell¹,

Librizzi²

2015

Pediatr Ann

View full text Add to dashboard Cite

A 5-year-old previously healthy girl presented to our hospital with 10 days of periorbital edema, abdominal pain, muscle weakness, and progressive fatigue. Four days prior to admission, her pediatrician prescribed diphenhydramine and fexofenadine for a possible allergic reaction, and polyethylene glycol 3,350 for constipation, but the patient showed no signs of improvement. She became progressively more fatigued, and 1 day prior to admission mild extremity edema was noted. The patient denied fever, vomiting, diarrhea, cough, joint pain or stiffness, recent rash, or trauma. She had visited Mexico 1 month prior. There was no significant family history. Outpatient testing revealed a negative urinalysis.In the emergency department, the patient was noted to be tachycardic (heart rate of 132 beats per minute) but otherwise well-appearing. Physical examination was significant for mild hepatomegaly, mild non-pitting edema of the bilateral upper and lower extremities, mild periorbital edema, and bilateral erythematous patches (2 cm × 2 cm) on her knees (Figure 1). Initial emergency department testing was significant for hypoalbuminemia (2.9 g/dL), transaminitis (aspartate aminotransferase [AST] of 231 units/L, alanine aminotransferase [ALT] of 139 units/L), and a mildly elevated erythrocyte sedimentation rate (24 mm/h).Initial differential diagnosis included autoimmune hepatitis, viral hepatitis, protein-losing enteropathy, and viral myocarditis. Coagulation studies and glucose challenge test were normal (suggesting normal synthetic liver function), hepatitis panel was negative, and the AST to ALT ratio of 231:139 made a primary hepatic etiology less likely. Sustained tachycardia prompted further cardiology testing; however, electrocardiogram and echocardiogram revealed normal cardiac function.Generalized fatigue and subjective muscle weakness remained a prominent symptom, and on further questioning the family then disclosed a history of intermittent facial rash and erythema above her eyelids (Figure 2). Therefore, a systemic inflammatory etiology was explored.

show abstract

Dermatomyositis presenting with severe subcutaneous edema. Five additional cases and review of the literature

Cited by 7 publications

References 0 publications

Perimysial microarteriopathy in dermatomyositis with anti‐nuclear matrix protein‐2 antibodies

Perimysial microarteriopathy in dermatomyositis with anti‐nuclear matrix protein‐2 antibodies

Prominent subcutaneous oedema as a masquerading symptom of an underlying inflammatory myopathy

A 5-Year-Old Girl with Fatigue, Diffuse Edema, and Weakness

Contact Info

Product

Resources

About